Severe intrahepatic cholestasis, erythrocytosis and hypoglycemia: unusual presenting features of systemic AL amyloidosis.
We report the case of a 68-year-old African woman who presented with jaundice, hepatomegaly and anasarca. Clinical investigation disclosed severe intrahepatic cholestasis, nephrotic syndrome, erythrocytosis and hypoglycemia. Diagnosis of systemic AL amyloidosis was established by percutaneous liver biopsy. Bone marrow biopsy showed 32% of myeloma cells. The patient started treatment with melphalan and prednisolone, but liver function deteriorated and she died in hepatic failure complicated by septic shock three weeks after the diagnosis. We present possible explanations for the unusual clinical and laboratory findings, which required a multidisciplinary approach and posed challenging problems in differential diagnosis and management.
Ferreira
Sara
S
Department of Gastroenterology, Liver Unit, Hospital de Santa Maria, E.P.E. Avenida Professor Egas Moniz, Lisbon, Portugal. saragferreira@netcabo.pt
Baldaia
Cilénia
C
Fatela
Narcisa
N
Ramalho
Fernando
F
Costa
Maria Adília
MA
Brito
Delfina
D
Esteves
Graça
G
Monteiro
Estela
E
D000368:Aged; D000686:Amyloidosis / Q000150:complications* / Q000175:diagnosis; D002780:Cholestasis, Intrahepatic / Q000175:diagnosis / Q000209:etiology*; D003937:Diagnosis, Differential; D004906:Erythrocyte Count; D017809:Fatal Outcome; D005260:Female; D006801:Humans; D007003:Hypoglycemia / Q000175:diagnosis / Q000209:etiology*; D019152:Paracentesis; D011086:Polycythemia / Q000175:diagnosis / Q000209:etiology*; D012720:Severity of Illness Index; D014057:Tomography, X-Ray Computed